In studies of GJB2-related hearing loss, the common pathologies are the failure of the tunnel of Corti (TC) to open and the disappearance of Nuel's space (NS), and these malformations of the organ of Corti (OC) have been observed both in humans and in transgenic mice (Griffith et al., 2006; Wang et al., 2009). Here, GJB2 is linked to hearing loss disorder.